(PACE 2009; 32:7-12) permanent pacemaker, cardiac surgery
IntroductionThe incidence of conduction disturbance requiring pacing after open heart operations is between 10% and 15%. [1][2][3][4] Most of these will recover, however, 1-3% of patients will require permanent pacemaker implantation. [5][6][7] We reviewed our experience with permanent pacemaker (PPM) implantation following cardiac surgery. Our aim was to identify predictors for PPM requirement and identify patients who will remain pacemaker dependent over long term.
Enzyme replacement therapy is the standard of care for symptomatic Gaucher disease. Velaglucerase alfa is a human -glucocerebrosidase produced in a wellcharacterized human cell line. A 9-month phase 1/2 open-label, single-center trial and ongoing extension study were conducted to evaluate safety and efficacy of velaglucerase alfa. Twelve symptomatic adult type 1 Gaucher patients (intact spleens) received velaglucerase alfa (60 U/kg per infusion) during phase 1/2. An extension study was offered to patients completing the trial; step-wise dose reduction (to 30 U/kg per infusion) was instituted. Eleven patients completed phase 1/2; 10 entered the extension; 9 patients reached 39 months of extension. No drug-related serious adverse events or withdrawals, and no antibodies were observed. Home therapy was successfully implemented during the extension. Statistically significant improvements (P < .004) were noted in mean percentage change from baseline to 9 months and baseline to 48 months for hemoglobin (؉19.2%, ؉21.7%, respectively), platelet counts (؉67.6%, ؉157.8%, respectively), normalized liver volume (؊18.2%, ؊42.8%, respectively), and normalized spleen volume (؊49.5%, ؊79.3%, respectively).
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